Neurosurg Focus
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OBJECTIVE Chordoma is a rare malignant tumor for which en bloc resection with wide margins is advocated as primary treatment. Unfortunately, due to anatomical constraints, en bloc resection to achieve wide or marginal margins is not feasible for many patients as the resulting morbidity would be prohibitive. The objective of this study was to evaluate the efficacy of intralesional curettage and separation surgery followed by spinal stereotactic body radiation therapy (SBRT) in patients with chordomas in the mobile spine. ⋯ CONCLUSIONS The combination of surgery and SBRT provides excellent local control following intralesional curettage and separation surgery for chordomas in the mobile spine. Patients who underwent intralesional curettage and spinal SBRT as initial treatment had better disease control than those undergoing salvage therapy. High-dose radiotherapy may offer several biological benefits for tumor control.
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OBJECTIVE An analysis of factors contributing to durable radiographic control of spinal metastases was undertaken, drawing from a large single-institution database in an attempt to elucidate indications and dose requirements for successful treatment. METHODS All patients treated at a single institution with stereotactic radiosurgery (SRS) of the spine as first-line therapy were assessed for local progression of the treated site, defined as radiographic enlargement of the treated tumor and/or biopsy-proven evidence of active tumor cells. All patients were followed with CT, PET, or MR imaging every 3-6 months until death. ⋯ Patients in the high-dose cohort had a 2% cumulative rate of local failure. Histological findings were not associated with local failure, suggesting that radioresistant histological types benefit in particular from radiosurgery. For patients with a favorable prognosis, a higher dose of SRS is important for long-term outcomes.
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Review
Molecular pathology of adamantinomatous craniopharyngioma: review and opportunities for practice.
Since the first identification of CTNNB1 mutations in adamantinomatous craniopharyngioma (ACP), much has been learned about the molecular pathways and processes that are disrupted in ACP pathogenesis. To date this understanding has not translated into tangible patient benefit. The recent development of novel techniques and a range of preclinical models now provides an opportunity to begin to support treatment decisions and develop new therapeutics based on molecular pathology. In this review the authors summarize many of the key findings and pathways implicated in ACP pathogenesis and discuss the challenges that need to be tackled to translate these basic science findings for the benefit of patients.
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OBJECTIVE Craniopharyngiomas remain a particularly formidable challenge in the neurosurgical field. Because these lesions involve the hypothalamus and ophthalmological systems, their resection is associated with either higher rates of mortality and recurrence or a lower rate of radical resection. The authors report the outcomes of aggressive surgeries based on an anatomical subclassification of craniopharyngiomas. ⋯ Overall, disease in 69 (95.8%) of 72 patients was well controlled at the last follow-up. CONCLUSIONS Aggressive tumor resection is the authors' treatment policy for craniopharyngioma. Using an anatomical subclassification of craniopharyngioma to choose the most appropriate surgical approach is helpful in achieving that goal of aggressive resection.
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The recent genomic and transcriptomic characterization of human craniopharyngiomas has provided important insights into the pathogenesis of these tumors and supports that these tumor types are distinct entities. Critically, the insights provided by these data offer the potential for the introduction of novel therapies and surgical treatment paradigms for these tumors, which are associated with high morbidity rates and morbid conditions. Mutations in the CTNNB1 gene are primary drivers of adamantinomatous craniopharyngioma (ACP) and lead to the accumulation of β-catenin protein in a subset of the nuclei within the neoplastic epithelium of these tumors. ⋯ The vast majority of PCPs harbor the BRAFv600e mutation. There are now 2 reports of patients with PCP that had dramatic therapeutic responses to targeted agents. Ongoing clinical and research studies promise to not only advance our understanding of these challenging tumors but to offer new approaches for patient management.